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. 2022 Feb 18;61(3):605-613.
doi: 10.1093/ejcts/ezab432.

Long-term outcomes in children undergoing vascular ring division: a multi-institution experience

Di Yu  1 , Zhangke Guo  2 , Xin You  3 , Wei Peng  1 , Jirong Qi  1 , Jian Sun  1 , Kaihong Wu  1 , Xiaofeng Li  2 , Xuming Mo  1
Affiliations

Long-term outcomes in children undergoing vascular ring division: a multi-institution experience

Di Yu et al. Eur J Cardiothorac Surg. .

Abstract

Objectives: Vascular rings are rare anomalies of congenital heart disease that cause respiratory and gastrointestinal symptoms. This study assessed the long-term outcomes of patients with vascular ring division.

Methods: A multi-institution retrospective review of 371 patients with vascular rings undergoing surgical division at 3 paediatric cardiac institutions between November 2007 and October 2019 was performed.

Results: The complete vascular rings consisted of a double aortic arch (24.5%), right aortic arch with left ligamentum arteriosum (36.7%) and left aortic arch, with right ligamentum arteriosum (0.5%). The incomplete vascular rings consisted of a pulmonary artery sling (22.9%), left aortic arch with aberrant right subclavian artery (15.1%) and innominate artery compression syndrome (0.3%). Respiratory symptoms included stridor (71.4%), wheezing (49.1%), coughing (31.5%), gastrointestinal symptoms included choking (12.4%), dysphagia (3.2%) and emesis (1.9%). Only one patient died after discharge, yielding a late mortality rate of 0.3% (1/360). The 10-year overall survival rate was 96.8%. Postoperative complications were reported in 51 patients, 15 of whom required reoperation. The 10-year freedom from reoperation rate was 95.9%. Follow-up was completed in 95.4% (354/371) of patients, with a mean follow-up time of 4.3 ± 2.9 years (range from 1 to 13 years). Twenty patients (5.6%) experienced residual symptoms during long-term follow-up.

Conclusions: The outcomes of vascular ring division are excellent. A Kommerell diverticulum >1.5 times the aberrant left subclavian artery origin is an operative indication for primary resection. Tracheomalacia is a risk factor for reoperation and residual symptoms, and preoperative fibrobronchoscopy is important for evaluation.

Keywords: Kommerell diverticulum; Surgery; Tracheal ring; Tracheomalacia; Vascular ring.

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Figures

Figure 1:
Figure 1:
The CONSORT diagram. ARSCA: aberrant right subclavian artery; DAA: double aortic arch; IACS: innominate artery compression syndrome; LAA: left aortic arch; LLA: left ligamentum arteriosum; PAS: pulmonary artery sling; RAA: right aortic arch; RLA: right ligamentum arteriosum.
Figure 2:
Figure 2:
Rate of overall survival and freedom from reoperation related to vascular ring repair. Y-axis is truncated at 0.9.
Figure 3:
Figure 3:
Patients with severe tracheomalacia treated with 3D-printed bioresorbable airway splints: (A) preoperatively and (B) 1 year postoperatively.
None
See this image and copyright information in PMC

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References

    1. Gross RE. Surgical relief for tracheal obstruction from a vascular ring. N Engl J Med 1945;233:586–90. - PubMed
    1. Backer CL, Mavroudis C, Rigsby CK, Holinger LD.. Trends in vascular ring surgery. J Thorac Cardiovasc Surg 2005;129:1339–47. - PubMed
    1. Lee EY, Boiselle PM, Shamberger RC.. Multidetector computed tomography and 3-dimensional imaging: preoperative evaluation of thoracic vascular and tracheobronchial anomalies and abnormalities in pediatric patients. J Pediatr Surg 2010;45:811–21. - PubMed
    1. Lambert V, Sigal-Cinqualbre A, Belli E, Planché C, Roussin R, Serraf A. et al. Preoperative and postoperative evaluation of airways compression in pediatric patients with 3-dimensional multislice computed tomographic scanning: effect on surgical management. J Thorac Cardiovasc Surg 2005;129:1111–8. - PubMed
    1. Backer CL, Mavroudis C.. Congenital Heart Surgery Nomenclature and Database Project: vascular rings, tracheal stenosis, pectus excavatum. Ann Thorac Surg 2000;69:S308–18. - PubMed